Characterizing visual processing deficits in cerebral adrenoleukodystrophy.

Autor: Corre CS; Department of Neurology, University of Rochester Medical Center, United States; Department of Neurology, Massachusetts General Hospital, United States., Bambery M; Department of Neurology, Massachusetts General Hospital, United States., Bennett CR; The Laboratory for Visual Neuroplasticity, Massachusetts Eye and Ear, United States., Nagy A; Department of Neurology, Massachusetts General Hospital, United States; Harvard Medical School, United States., Manley CE; Department of Neurology, Massachusetts General Hospital, United States., Winter E; Department of Neurology, Massachusetts General Hospital, United States., Peregoy C; Department of Neurology, Massachusetts General Hospital, United States., Kelly D; Department of Neurology, Massachusetts General Hospital, United States., Andonian H; Department of Neurology, Massachusetts General Hospital, United States., Maciel S; Department of Neurology, Massachusetts General Hospital, United States., Becker C; Department of Neurology, Massachusetts General Hospital, United States., Merabet LB; The Laboratory for Visual Neuroplasticity, Massachusetts Eye and Ear, United States; Harvard Medical School, United States., Eichler FS; Department of Neurology, Massachusetts General Hospital, United States; Harvard Medical School, United States. Electronic address: feichler@mgb.org.
Jazyk: angličtina
Zdroj: Brain & development [Brain Dev] 2024 Nov; Vol. 46 (10), pp. 344-350. Date of Electronic Publication: 2024 Oct 12.
DOI: 10.1016/j.braindev.2024.09.008
Abstrakt: Background and Objectives: Cerebral adrenoleukodystrophy (CALD) can cause visual impairment, but early symptoms are often missed or misdiagnosed. The framework of cerebral visual impairment (CVI) distinguishes deficits in sensory detection ("lower order") from those of perception and interpretation ("higher order"). This study describes visual deficits in patients with CALD and higher order visual function assessed with a virtual reality (VR) interface combined with eye tracking.
Methods: A retrospective medical record review assessed the prevalence of visual deficits in patients with CALD, as well as lesion burden on brain MRI using the Loes MRI severity score. A VR-based task measured visual spatial processing performance in participants with CALD and controls.
Results: Out of 89 CALD patients, 69 % had at least one sign or symptom of visual impairment. Lower order deficits were seen in 56 % of patients, and higher order deficits were seen in 59 % of patients who underwent neuropsychological testing. Even in early stage disease (Loes MRI severity score ≤ 3), visual impairment was present in more than half of patients (58 %). On prospective VR-based assessment, the CALD group (n = 30) had impaired visual search performance (lower success rate and longer reaction time) compared with controls (n = 38). In both groups, there was a trend of worsening performance with increasing task difficulty.
Discussion: Higher order visual deficits, not just impairment of visual acuity, visual fields, or oculomotor function, are common in all stages of CALD. Beyond neuropsychological testing, VR-based functional testing allows for quantitative assessment of higher order visual perceptual deficits that are relevant to everyday tasks and may serve as an important marker of neurological decline.
Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have influenced or appeared to influence the work reported in this paper.
(Copyright © 2024. Published by Elsevier B.V.)
Databáze: MEDLINE
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